Long-Term Effect of Sirolimus on Serum VEGF-D Levels in Patients with Lymphangioleiomyomatosis

Background

Sirolimus reduces serum levels of VEGF-D, size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas, and stabilizes lung function in patients with lymphangioleiomyomatosis.

Methods

To determine whether reductions in VEGF-D levels are sustained over time, and parallel changes in lung function and lymphatic disease, we evaluated 25 patients with lymphangioleiomyomatosis and measured VEGF-D levels, lung function and extent of lymphatic disease before and during sirolimus therapy

Results

Treatment with sirolimus stabilized FEV1 and DLCO over a period of 4.5±1.6 years, caused resolution of lymphatic disease, and reduced angiomyolipomas size and VEGF-D levels (3,720±3,020 to 945±591 pg/ml, pConclusions

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